乳头状胶质神经元肿瘤2例临床病理观察及文献复习

Papillary glioneuronal tumors:A clinicopathological observation of 2 cases and literature review

目的:探讨乳头状胶质神经元肿瘤(papillary glioneuronal tumors,PGNT)的临床病理特征、诊断、治疗及预后。方法:收集2例PGNT的临床病理资料,观察其组织病理学特征及免疫表型,并复习相关文献。结果:大体切面例1患者切面灰黄,质软,例2患者灰黄间褐,囊实性,质稍软。两例肿瘤镜下表现为扁平和立方状的星形细胞围绕透明变性血管,排列成假乳头状结构,乳头状结构间可见片状分布的神经元细胞及神经节样细胞,部分区域坏死出血,局灶可见少许含铁血黄素沉积。其中一例细胞可见轻度非典型性。两例患者免疫组化结果相同,均显示GFAP、S-100、VIM、NeuN、Syn、olig-2(+),CK、EMA、CD34均(-),Ki-67 LI 1%~2%。结论:PGNT是近年来提出的新分类肿瘤,病例报道较少,PGNT在绝大多数病例中是惰性的,对治疗有良好的反应,但罕见的非典型存在具有一定的警示性,需要更长的随访,以进一步阐明其生物学行为。其特征性的组织形态、免疫组化表型有助于诊断和鉴别诊断。

Objective:To investigate the clinicopathological features,diagnosis,treatment and prognosis of papillary glioneuronal tumors(PGNT).Methods:The clinicopathological data of 2 cases of PGNT were collected,the histopathological features and immunophenotype were observed,and the related literatures were reviewed.Results:In general,one case was grey yellow and soft,and the other case was gray brown,with solid cystic nature and slightly soft substance. Microscopically,the tumors showed flat and cuboidal stellate cells arranged around the hyaline and degenerative vessels. Tumor cells arranged in a pseudopapillary structure,between the papillary structure can be seen sheet-like distribution of neurons and ganglion-like cells. Some areas were necrotic and hemorrhagic,and there was little deposition of hemoflavin in the foci. One case showed mild atypia. The immunohistochemical results of the two patients were the same,showing that the tumor cells were positive for GFAP,S-100,VIM,NeuN,Syn,Olig-2,and negative for CK,EMA,CD34. Ki-67 LI was about 1%~2%. Conclusion: PGNT is a newly classified tumor proposed in recent years. There are few cases reported. PGNT is inert in most cases and has a good response to treatment. However,rare atypical tumors have a certain warning and require longer follow-up to further clarify their biological behavior.Its characteristic histomorphology and immunohistochemical phenotype are helpful for diagnosis and differential diagnosis.